Efficacy of combination treatment with cyclosporin A and corticosteroids for acute interstitial pneumonitis associated with dermatomyositis

Interstitial pneumonitis (IP) associated with polymyositis and dermatomyositis (PM/DM) is a serious complication that affects prognosis. We therefore undertook a retrospective multicenter study to examine the efficacy of a combination treatment with cyclosporin A (CsA) and corticosteroids. Fifty-three IP patients with PM/DM (9 PM, 44 DM) were analyzed. Thirty-two patients treated with CsA plus corticosteroids (9 PM, 23 DM) were included in the study. Four parameters, i.e., subjective symptoms, ausculatory sound, chest radiographs, and respiratory index, were serially evaluated. A general evaluation was performed 4 weeks after the start of the combination treatment. All patients with PM and chronic IP with DM, and 52% of those with acute IP with DM were graded as better than partially effective in the general evaluation. In contrast, all patients graded as progressive in the general evaluation had acute IP with DM. It is of note that in acute IP with DM, the survival rate of the group primarily treated with CsA and corticosteroids from the early stage of their disease was significantly higher than that of the group initially treated with corticosteroids alone (P = 0.049). In conclusion, a combination treatment of CsA and corticosteroids from the early stage of disease may be advantageous for patients with IP with PM/DM, especially acute IP with DM.     

Nation-wide survey for the treatment with cyclosporin A of interstitial pneumonia associated with collagen diseases]

OBJECTIVES: This study was performed to investigate the efficacy and safety of cyclosporin A (CsA) for the treatment of interstitial pneumonia (IP) associated with collagen diseases in Japan. METHODS: Questionnaires were sent to 36 hospitals specializing in collagen diseases. RESULTS: Fifty-eight patients (7 polymyositis (PM), 19 dermatomyositis (DM), 7 systemic sclerosis (SSc), 7 rheumatoid arthritis (RA), 2 mixed connective tissue disease (MCTD), 1 systemic lupus erythematosus (SLE) and 1 Sj?gren's syndrome (SS), 1 RA + SSc, 2 PM + SSc, 1 DM + SLE, and 10 idiopathic interstitial pneumonia (IIP) with IP were treated with CsA at 14 hospitals. IP was classified into the acute or chronic type. In the PM/DM group (7 PM, 19 DM, 2 PM + SSC, 1 DM + SLE), 65.5% were the acute type. In the other collagen disease group (7 SSc, 7 RA, 2 MCTD, 1 SLE, 1 SS, and 1 RA + SSc) and IIP group, 36.8% and 50% were the acute type, respectively. Mean dosages of CsA were 3.7 +/- 1.3 mg/kg/day for the PM/DM group, 3.0 +/- 1.0 for the other collagen disease group, and 3.8 +/- 4.8 for the IIP group. Oral corticosteroids were administered in combination with CsA in 100, 73.7, and 70% of the patients with PM/DM, other collagen disease, and IIP groups, respectively. CsA was effective for 72.2, 33.3, and 25% of the acute IP cases in the PM/DM, other collagen disease, and IIP groups, respectively. CsA was effective for 50.0, 50.0, and 60.0% of chronic IP cases in the PM/DM, other collagen disease, and IIP groups, respectively. Twenty-three adverse effects were observed, but most of them ameliorated upon withdrawal or reduction of the CsA dose. CONCLUSION: CsA is effective for the treatment of acute type IP associated with collagen diseases, especially PM/DM. To perform a prospective multi-center trial, standards for the recruitment of patients, efficacy assessments, and trial course and treatment should be determined carefully.     

Clinical study of 10 cases of acute or subacute interstitial pneumonia associated with dermatomyositis

The prognosis for dermatomyositis (DM) with acute interstitial pneumonia (IP) is very poor. In the past 5 years, we have treated 10 DM patients with acute or subacute IP. Six cases were of acute-type IP, and 4 were of subacute-type IP. The treatment was a combination therapy of methylprednisolone (m-PSL) pulse therapy, cyclophosphamide (CPA) pulse therapy, oral cyclosporine A (CsA), and oral PSL. The outcome was 5 deaths and 5 survivals. All 5 cases of death had acute-type IP, four of which were complicated with pneumomediastinum, and these patients died within 40 days of IP onset. Furthermore, 4 of the 5 death cases were diagnosed with amyopathic DM, and one had hypomyopathic DM. The survivors comprised one case of acute-type IP with marked myositis, and 4 subacute cases. These results suggested that the prognosis for DM with IP might be dependent on the type of IP, the severity of the myositis, and the existence of pneumomediastinum. The rapid establishment of a more useful diagnostic technique and therapy for early-phase DM with acute IP is hoped for.     

Cyclosporin treatment in steroid-resistant and acutely exacerbated interstitial pneumonia

OBJECTIVE: The aim of this study was to evaluate the efficacy of cyclosporin A (CsA) in patients with interstitial pneumonia (IP). DESIGN: Retrospective comparative study. PATIENTS: We reviewed 33 patients (23 males and 10 females with a mean age of 62.5 years) with histologically-proven progressive IP who were treated with CsA. All patients had corticosteroid-resistant IP or developed acute exacerbation of IP in their courses. RESULTS: The underlying systemic diseases were: idiopathic interstitial pneumonias (IIPs) in 19 patients, and collagen vascular diseases (CVDs) in 14. The histopathological patterns and underlying diseases of IP were classified as usual interstitial pneumonia (UIP)/idiopathic pulmonary fibrosis (IPF) in 10 patients, cellular-nonspecific interstitial pneumonia (NSIP)/IIPs in 3, fibrotic-NSIP/IIPs in 5, organizing pneumonia (OP)/IIP in 1, UIP/CVDs in 4, cellular-NSIP/CVDs in 7, fibrotic-NSIP/CVDs in 2, and diffuse alveolar damage (DAD)/CVD in 1, respectively. They received a low dosage of CsA combined with corticosteroids. The prognoses after treatment with CsA were well correlated with histopathological patterns. Cellular-NSIP and OP showed better prognoses than fibrotic-NSIP, UIP or DAD. In addition, CVDs had better prognoses than IIPs, when compared on the basis of the same histopathological patterns. Furthermore, the prognoses in the CsA-treated group were significantly better than in those without CsA treatment in regard to acute exacerbation of UIP/IPF. CONCLUSIONS: CsA combined with corticosteroids may be an efficacious treatment for corticosteroid-resistant IP and for acute exacerbation of IPF.     

Corticosteroid resistant interstitial pneumonitis in dermatomyositis/polymyositis: prediction and treatment with cyclosporine.

OBJECTIVE: To determine the characteristics of corticosteroid resistant interstitial pneumonitis (IP) in dermatomyositis (DM) and polymyositis (PM), and to evaluate the effect of cyclosporine on corticosteroid resistant IP in DM/PM. METHODS: We analyzed retrospectively the incidence, clinical features, and corticosteroid responses of IP in 111 patients with DM (56) or PM (55). All patients with DM/PM were treated with prednisolone, and corticosteroid resistant IP was defined as a progression of IP despite administration of 1 mg/kg/day prednisolone for more than 4 weeks. We also evaluated the effect of cyclosporine on corticosteroid resistant IP in patients with DM/PM. RESULTS: IP occurred in 24 of 56 DM and 12 of 55 PM patients. We then classified IP in DM/PM according to serum CPK levels at the onset of IP; IP associated with high CPK levels (type I) (19) and IP associated with normal CPK levels (type II) (17). Only 2 of 19 (11%) type I IP were resistant to prednisolone therapy, while 14 of 17 (82%) type II IP were resistant to prednisolone therapy. Thus, patients with type II IP showed poorer prognosis than those with type I IP (one year survival rate: type I 89% vs type II 31%). Cyclosporine was effective in all 5 cases with corticosteroid resistant IP in DM/PM (one year survival rate 80%). CONCLUSION: (1) Corticosteroid resistant IP develops mostly in patients with DM/PM without CPK elevation at the onset of IP (type II IP), and (2) cyclosporine is effective for the corticosteroid resistant IP in DM/PM and significantly prolongs survival of patients.     

Cyclosporin A therapy for interstitial pneumonitis associated with rheumatic disease

To determine the efficacy of cyclosporin A (CysA) for the treatment of steroid-resistant interstitial pneumonitis (IP), we enrolled 25 patients with various rheumatic diseases and steroid-resistant IP in a pilot study [4 patients with rheumatoid arthritis (RA), 2 with systemic lupus erythematosus (SLE), 11 with polymyositis/dermatomyositis (PM/DM), 4 with systemic sclerosis (SSc), 1 with mixed connective tissue disease (MCTD), 3 with Sjögren syndrome (SS)]. Twelve patients (48%) showed a persistent response to CysA therapy, and 7 of them had PM/DM, including so-called amyopathic DM. Patients with a persistent response had moderately elevated lactate dehydroxygenase (LDH) levels, whereas patients who died had much higher LDH levels and hypoxia. Even patients with low blood levels of CysA achieved a persistent response. In responding patients, the symptoms, chest X-ray findings, arterial oxygen tension, and LDH level all improved after less than 4 weeks. In conclusion, CysA seem to be useful for treating patients with steroid-resistant IP, whose duration is short and severity is mild.     

Prospective study of high-dose intravenous immunoglobulin for the treatment of steroid-resistant polymyositis and dermatomyositis

Abstract

High-dose intravenous immunoglobulin (IVIG) therapy has been effective in treating many autoimmune and systemic inflammatory diseases. In the present prospective study, we evaluated the efficacy of IVIG for patients with polymyositis (PM) and dermatomyositis (DM) refractory to treatment with high-dose corticosteroids. PM/DM was defined as steroid-resistant when the muscle strength of a patient did not improve despite the administration of more than 50?mg prednisolone per day for more than 4 weeks. A total of 12 patients with biopsy-proven, steroid-resistant PM/DM received one infusion of polyethylene glycol-treated human IgG at a dose of 0.4?g per kg per day for five successive days. Three of the patients received a second infusion. All patients were followed for up to 3 months after the infusion. Finally, 8 patients (6 PM and 2 DM; 5 men and 3 women) aged 29–67 years (mean 48 years) were analyzed. Their clinical response was assessed by changes in (a) subjective signs, i.e., fatigue (visual analog scale, VAS), muscle pain (VAS), activities of daily living (ADL), (b) objective signs, i.e., manual muscle strength (MMT) and serum level of creatine kinase (CK). At 12 weeks after the infusion, the patients showed significant improvement in their scores of muscle strength (from a mean of 67.0 to 81.0) and their ADL scores (from a mean of 27.1 to 39.1). The mean serum CK level decreased significantly from 1287.4 to 612.6?IU/l. In addition, the mean VAS of fatigue decreased significantly from 5.5 to 1.3?cm. The physicians’ assessment showed that 87.5% of patients had improved. The average reduced dose of prednisolone was 47.1?mg/day at 12 weeks after infusion in 7 patients who exhibited improvement. Adverse effects, i.e., asymptomatic myocardial infarction and increased blood urea nitrogen (BUN), were noted with two of the 15 infusion (13%). Overall, IVIG was found to be safe and effective for refractory PM and DM.     

Cyclosporin A treatment of interstitial pneumonia]

To assist in making prognoses for patients with interstitial pneumonia (IP) who were treated with cyclosporin A (CsA), we conducted a review of forty-nine patients (32 men and 17 women with a mean age of 62 yrs) with progressive IP during the period from 1997 through 2001. All patients were steroid-resistant or acutely exacerbated cases. They received a low dosage of CsA (100-130 mg/day) combined with corticosteroids. Before and after the CsA therapy, blood gas analysis and HRCT scans were evaluated. Twenty-five patients underwent video-assisted thoracoscopic surgery (VATS) or autopsy for a histopathological evaluation. Among the 49 patients with IP, the documented underlying systemic diseases were of unknown etiology (IPF or IIP) in 26 and were collagen vascular diseases (CVD) in 23. The chest CT pattern and underlying systemic diseases of IP were classified as a usual interstitial pneumonia (UIP) pattern/IPF in 16 cases, a non-UIP pattern/IIP in 10 cases, a UIP pattern/CVD in 7 cases, and a non-UIP pattern/CVD in 16 cases. The prognoses after CsA treatment were improved or unchanged in 27% of cases with a UIP pattern/IPF, 78% of cases with a non-UIP pattern/IIP, 71% of cases with a UIP pattern/CVD and 75% of cases with a non-UIP pattern/CVD; deteriorated in 73%, 22%, 29% and 25% of cases, respectively, with these patterns and underlying diseases. At present, four out of thirteen (31%) patients with acute exacerbation of UIP pattern/IPF have survived for four to twelve months (mean: 7.5 months). Four patients revealed re-exacerbation of IP after the dose of CsA was tapered. Among the 25 patients with IP, the histopathological patterns of IP were classified as usual interstitial pneumonia (UIP) in 10 cases, nonspecific interstitial pneumonia (NSIP) in 14 cases (group I, 2; group II, 5; group III, 7) and diffuse alveolar damage (DAD) in 1 case. The prognoses were improved or unchanged in all cases of NSIP group I, in 80% of cases with NSIP group II, in 29% of cases of NSIP group III and in 20% of cases of UIP; and deteriorated in the case of DAD, in 80% of cases of UIP, in 71% of cases of NSIP group III, and in 20% of cases of NSIP group II. It should be emphasized that CsA combined with corticosteroids may be effective for the treatment of steroid-resistant or acute exacerbation cases of IP. Further studies are required to determine long-term outcome with this treatment.     

Cyclosporin-induced cortical blindness in a patient with dermatomyositis

Abstract

Cyclosporin A (CsA) has various adverse effects including neurotoxicity. We report the case of a 49-year-old Japanese woman with dermatomyositis who showed CsA-induced cortical blindness. The patient demonstrated cortical blindness despite having a normal blood concentration of CsA. The risk factors of CsA-induced cortical blindness include the use of corticosteroids and vascular injury, which are frequently observed in patients with systemic autoimmune diseases. Clinicians should consider CsA neurotoxicity when using CsA for patients with systemic autoimmune diseases.     

A case of dermatomyositis with “liver disease associated with rheumatoid diseases” positive for anti-liver–kidney microsome-1 antibody

We reported a case of dermatomyositis (DM) with liver disturbance in a 50-year-old Japanese female. She presented with fever, muscle weakness, and typical DM rashes. On clinical and serological examinations, the liver impairment was initially diagnosed as probable autoimmune hepatitis, which was denied by a histological study despite positive anti-liver–kidney microsome-1 antibody. Finally, she was diagnosed as having DM with “liver disease associated with rheumatoid diseases”, and treatment with oral prednisone (40 mg/day) achieved normalization of liver and muscle enzyme levels as well as improvement of symptoms associated with DM. Liver involvement in patients with polymyositis (PM)/DM has not been well described and is considered to be uncommon. Full clarification of the etiology of liver impairment with a histological examination in collagen diseases including PM/DM is useful to determine the proper dose of corticosteroids for the treatment of collagen diseases and their liver complications.     

Overview of dietary supplements on patients with type 2 diabetes

Background and aimsThe primary approach for managing type 2 diabetes mellitus (T2DM) involves lifestyle modification and diet therapy along with pharmacologic interventions. Many patients are interested to identify nutritional supplements that may provide benefit in prevention and management of diabetes. However, the efficacy and safety of nutritional supplements such as chromium, n-3 polyunsaturated fatty acids (PUFAs), vitamin D, zinc and magnesium in disease treatment is a worrying and controversial matter. In this narrative review, patients and health care providers are introduced to the effects of mentioned dietary supplements that may help in choosing or not choosing these supplements in treatment of diabetes.MethodsThis review was carried out using comprehensive and systematic literature reports on the dietary supplements in the management of diabetes. Empirical searches were conducted using Google Scholar, Science Direct and PubMed databases. Searches were also undertaken using keywords, in English, such as “chromium” OR “vitamin D” OR “omega-3 fatty acids” OR “zinc” OR “magnesium” in combination with “type 2 diabetes”.ResultsThe available evidence is insufficient to create a definite conclusion that nutritional supplements including chromium, n-3 PUFAs, vitamin D, zinc and magnesium might be beneficial for the prevention and treatment of T2DM and therefore, the general recommendation to use these supplements in the management of diabetes cannot be justified. The results of most studies lack uniformity across multiple aspects, including different dose and formation of supplements, duration, and subjects under intervention.ConclusionsThere is a need for well-designed, high quality, large and long-term studies to strengthen the available evidence and ensure the safety and efficacy of products.     

Medical audit on children with asthma in an Emergency Department

ObjectiveTo carry out a medical audit or evaluation and improvement procedure on the management of children with asthmatic crises in our Emergency Department (ED).Material and methodsWe carried out a retrospective audit between January and March 2007, analysing the medical records of a random sample of 50 patients aged 2–14 years consulting our ED for asthmatic crises. Following the international guides, we first selected 17 explicit indicators divided into four domains: “evaluation”, “examination”, “diagnostic resources”, and “treatment and conditions at discharge”.ResultsIndicators’ compliance proved unequal; it was scarce for cause of asthma crisis (32%); degree of severity (18%); and supportive treatment (24%). Auscultation was registered in 100%, but respiratory frequency only in 49%, and peak flow in 0%. A total of 78% of the patients were treated in the ED, in all cases with beta-mimetic agents, and with systemic corticosteroids in 12%. The result of treatment was registered in only 69% of cases. The medical documentation of resident doctors was not signed by the staff.ConclusionsWe identified the following weak points: failure to determine the degree of severity; lack of specification of the details of the crisis (prior duration, treatment at home, supportive treatment); scant asthma background history; and deficient recording of respiratory frequency and peak flow. We propose improving the anamnesis, recording respiratory frequency, with the introduction of tools to measure peak flow, specification of treatment response, and the development of a simpler and more practical protocol, with the performance of a re-audit.     

Dermatomyositis, complicated with pneumomediastinum, successfully treated with cyclosporine A: a case report and review of literature

We report a rare case of patient with dermatomyositis (DM) who developed spontaneous pneumomediastinum (PnM) and subcutaneous emphysema. She was successfully treated with oral prednisolone and cyclosporine A (CsA). We reviewed the cases of PnM in patients with DM treated with CsA. A review of four previously reported cases revealed that treatment with systemic glucocorticoid and CsA was effective for the DM and PnM. We indicate that initial and early treatment of the patients with DM and PnM with CsA enabled rapid tapering of the dose of glucocorticoid and improved the disease.     

Pregnancy outcomes in adult patients with dermatomyositis and polymyositis

ObjectiveThe idiopathic inflammatory myopathies dermatomyositis (DM) and polymyositis (PM) are autoimmune diseases that can affect females of childbearing potential. We assessed pregnancy outcomes in DM and PM patients compared with the general obstetric population.MethodsThe Nationwide Inpatient Sample (NIS) (1993–2007) was used to identify delivery-associated hospitalizations in women with DM or PM (DM/PM, n = 853). Controls were from the general obstetric population delivery-associated hospitalizations matched to each case by year of delivery. Pregnancy outcomes included hospital length of stay (LOS), hypertensive disorders (HTN), premature rupture of membranes (PROM), intrauterine growth restriction (IUGR), and cesarean delivery. Multivariate regression analyses were performed using maternal age, race/ethnicity, and diabetes mellitus as covariates.ResultsOn multivariate analysis, patients with DM/PM had longer LOS compared to controls (p < 0.001). DM/PM was associated with an increased risk of hypertensive disorders compared to controls (OR = 2.90, 95% CI: 2.00–4.22). There were no differences in rates of PROM, IUGR, or cesarean section in patients with DM/PM compared with controls. Independent of a DM/PM diagnosis, African-American race, older age, and diagnosis of diabetes increased the hospital LOS (p < 0.001). African-American race and diabetes increased the risk of hypertensive disorders (OR = 1.38, 95% CI: 1.19–1.60; OR = 2.94, 95% CI: 2.04–4.23, respectively) compared to controls.ConclusionThese data suggest that patients with inflammatory myopathies are at increased risk of hypertensive disorders of pregnancy and longer length of hospitalization. Vigilant monitoring of blood pressure is advisable in pregnant patients with DM or PM.     

Morbidity and Mortality in Adult Polymyositis and Dermatomyositis

Before the use of corticosteroids, the prognosis for polymyositis/dermatomyositis (PM/DM) was extremely poor. To date, although overall prognosis appears to be better, PM and DM are still considered to be associated with increased morbidity, primarily related to severe muscle weakness and visceral involvement. Recent series underline that only 20% to 40% of treated patients will achieve PM/DM remission, whereas 60% to 80% will experience a polycyclic or chronic, continuous course of the disease. PM/DM further continues to have a great impact on life in medium- and long-term follow-up, as up to 80% of treated patients are still disabled (using Health Assessment Questionnaire scores). The overall mortality ratio in PM/DM patients also remains threefold higher compared with the general population, with cancer, lung, and cardiac complications and infections being the most common causes of deaths. Predictive factors for a poor prognosis in PM/DM patients are older age, involvement of lung and cardiac systems, dysphagia, cancer, and serum myositis–specific antibodies (including coexistent presence of anti-Ro52 and anti-Jo1 antibodies, anti–signal recognition particle antibody, anti-155/140, and anti–CADM-140 antibodies).     

Early intervention with corticosteroids and cyclosporin A and 2-hour postdose blood concentration monitoring improves the prognosis of acute/subacute interstitial pneumonia in dermatomyositis

OBJECTIVE: We retrospectively examined the effect of combination therapy with prednisolone and cyclosporin A (CSA) for dermatomyositis (DM) presenting with acute/subacute interstitial pneumonia (A/SIP), the daily CSA dose, and the time from diagnosis of A/SIP to initiation of CSA treatment. METHODS: Subjects were 16 DM patients with A/SIP. Seven patients were treated initially with 1 mg/kg/day prednisolone. When IP was progressive, CSA was added (Group A). Nine patients were treated initially with 1 mg/kg/day prednisolone and 4 mg/kg/day CSA, and 2-h postdose blood concentration (C2) monitoring was used to maintain the serum CSA level at 1000 ng/ml (Group B). RESULTS: Four of 7 patients in Group A (57%) and 1 of 9 patients in Group B (11%) died of respiratory failure related to IP (p = 0.06). Combination therapy with prednisolone and CSA at >or= 200 mg/day initiated within 15 days of diagnosis was effective for treatment of DM-A/SIP. The trough level (C0) and daily CSA dose were higher in Group B (201.3 ng/ml and 200.0 mg/day, respectively) than in Group A (140.0 ng/ml and 166.4 mg/day). CSA was continued in all patients without severe side effects. No patient died of infection. CONCLUSION: Combination therapy of corticosteroids and CSA should be initiated during the early stage of IP. The daily CSA dose should also be controlled with measurement of serum CSA concentration to achieve maximal immunosuppressive effect. C2 monitoring is a useful tool for this control.     

Cyclosporin A followed by the treatment of acute exacerbation of idiopathic pulmonary fibrosis with corticosteroid

OBJECTIVE: Idiopathic pulmonary fibrosis (IPF) is a slowly progressive disease with a poor prognosis. Acute exacerbation is the worst stage in the clinical course of IPF, as the condition is unresponsive to most conventional therapies. Corticosteroids and other immunosuppressive drugs have been attempted for the treatment of acute exacerbation, but only with very limited effectiveness. This study was performed to examine the effect of cyclosporin A (CsA) on acute exacerbation of IPF. PATIENTS AND METHODS: Thirteen patients with acute exacerbation of IPF were retrospectively studied. All 13 patients received pulse-therapy with methylprednisolone (1,000 mg per day for 3 days), followed by oral prednisolone (40-60 mg per day). Seven patients were received CsA (1.0-2.0 mg/kg per day) after the treatment with corticosteroids. We attempted to keep the blood trough level of CsA between 100 and 150 ng/ml. RESULTS: Among the 7 patients treated with CsA, 4 patients have survived for 60, 120, 276 and 208 weeks, respectively; 2 did not respond to pulse-therapy with methylprednisolone and died within 8 weeks after the start of CsA treatment. The other patient experienced re-exacerbation and died 87 weeks after the discontinuation of CsA due to the development of viral encephalitis. In contrast, all 6 patients treated without CsA died within 66 weeks after the onset of acute exacerbation. Four of these patients responded to pulse-therapy with methylprednisolone, but their condition deteriorated again while the subsequent prednisolone was being tapered. CONCLUSION: CsA seems to prevent re-exacerbation of IPF and improve the patients' chances for long-term survival.     

Step-up versus primary intensive approach to the treatment of interstitial pneumonia associated with dermatomyositis/polymyositis: a retrospective study

Corticosteroids (CS) are the standard initial treatment for interstitial pneumonia (IP) associated with dermatomyositis (DM)/polymyositis (PM). However, many patients fail to respond and have significantly high mortality even if immunosuppressive drugs (ISDs) are subsequently added, while a more intensive initial approach using ISDs is suggested to improve their survival. We conducted a retrospective study to examine the association between initial therapeutic approaches and clinical outcomes of active IP in DM/PM patients. We reviewed medical records of 34 consecutive DM/PM patients who had active IP defined by the presence of pulmonary function abnormality or active symptoms, and compared clinical outcome between those patients to whom ISDs were added if CS alone did not result in a favorable response (a step-up approach) and those who were started on ISDs simultaneously with CS (a primary intensive approach). Clinical endpoints were death, pulmonary death, and progression or improvement of pulmonary function. The step-up approach was used in 20 patients, to 11 of whom ISDs were eventually added after a median of 2.0 weeks, while the primary intensive approach was used in 14 patients. The primary intensive approach group had significantly better survival than the step-up approach group (P = 0.030 by the log-rank test). These two groups did not differ significantly in demographic characteristics and baseline clinical and laboratory features. Intensive approach by starting ISDs simultaneously with CS in the initial treatment for active IP in DM/PM patients was associated with better survival, emphasizing the impact of initial treatment on their survival. Prospective clinical investigation of this approach is now needed, but the limited clinical utility of CS as an initial treatment might ethically challenge clinical-trial designing.     

Adverse impact of interstitial pulmonary fibrosis on prognosis in polymyositis and dermatomyositis

Interstitial pulmonary fibrosis occurs in approximately 9% of patients with PM/DM, yet its effect on the course of PM/DM has been scarcely noted. In this report, two patients with PM/DM and IPF were presented to highlight the fact that pulmonary disease can overshadow the primary muscle disorder and progress despite therapy with corticosteroids and nonsteroidal immunosuppression. Our patients were added to previously reported cases and an overview of PM/DM and IPF was presented. Sixty-seven patients with the diagnosis of PM/DM and IPF, with a mean age of 60 +/- 18 years, were identified. Pulmonary complaints were present in 64 cases. Fever was present in 18, arthritis or arthralgias in 11, and Raynaud's phenomenon in 9. Forty percent died after followup of 31 +/- 32 months. This mortality was significantly higher (P less than .05) than that in 745 historical controls with PM/DM without IPF. Progressive IPF was the immediate cause of death in 58% of those who died. A subgroup of 29 patients who had histologic documentation of both myositis and IPF had a mortality of 62% after 22 +/- 25 months. In this latter group, six patients had RP; five of these died. Patients who were not treated with corticosteroids also appeared to fare worse but, given the small number of patients involved, definite conclusions cannot be drawn. We conclude that patients with PM/DM can be adversely affected by the presence of IPF and that this negative impact may be exaggerated by RP and perhaps by lack of treatment.(ABSTRACT TRUNCATED AT 250 WORDS)     

Successful treatment using high-dose intravenous immunoglobulin in a patient with rapidly progressive interstitial pneumonia associated with dermatomyositis

A 36-year-old male patient with dermatomyo-sitis (DM) associated with rapidly progressive interstitial pneumonia (IP) was successfully treated by high-dose intravenous immunoglobulin (IVIG). He suffered from myopathy, skin lesions, and IP. In spite of the treatment with a high-dose corticosteroid, IP progressed rapidly. Then high-dose intravenous immunoglobulin (20 g/day, 4 days) was administered. The skin lesions, myopathy, and pulmonary lesions improved. High-dose IVIG was considered to be a relatively safe and effective treatment for progressive IP associated with DM. Received: October 9, 1999 / Accepted: May 24, 2000